childhood nephrotic syndrome - bc children’s

46
Childhood Nephrotic Syndrome A PARENT S HANDBOOK Developed by Pediatric Nephrology Program British Columbia Children’s Hospital British Columbia Provincial Renal Agency Vancouver, British Columbia, Canada 3 RD EDITION OCTOBER 2016

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Page 1: Childhood Nephrotic Syndrome - BC Children’s

ChildhoodNephrotic Syndrome

A PARENT’S HANDBOOK

Developed by

Pediatric Nephrology ProgramBritish Columbia Children’s Hospital

British Columbia Provincial Renal Agency

Vancouver, British Columbia, Canada

3RD EDITION • OCTOBER 2016

Page 2: Childhood Nephrotic Syndrome - BC Children’s

INFORMATIO

N ?When and how to contact us

If you think your child needs to be seen or if they are having problemswe recommend that you first try to contact your family doctor orpediatrician. If you are not able to reach your doctor, you can contactthe Renal Program at BC Children’s Hospital.

During business hours, please call 604 875 2272 and we will connectyou with one of the team members.

Outside business hours, please call the hospital switchboard at604 875 2345 and ask for the kidney doctor (nephrologist) on call.

Page 3: Childhood Nephrotic Syndrome - BC Children’s

Childhood Nephrotic Syndrome—A Parent’s Handbook

Developed byPediatric Nephrology Program

British Columbia Children’s Hospital

British Columbia Provincial Renal Agency

Vancouver, British Columbia, Canada

Members of the Clinical PathwayDevelopment Team

Dr. Alanoud Alshami, Pediatric Nephrology Fellow

Marisa Catapang, Clinical Research Coordinator

Dr. Rob Humphreys, Pediatric Nephrologist

Dr. Jasper Jöbsis, Pediatric Nephrology Fellow

Dr. Cherry Mammen, Pediatric Nephrologist

Dr. Douglas Matsell, Pediatric Nephrologist

Nonnie Polderman, Pediatric Renal Dietitian

Dr. Matt Sibley, Pediatric Resident

In consultation with

Dr. Jean-Pierre Chanoine, Pediatric Endocrinologist

Kathleen Collin and Kathryn Haubrich, Pediatric Pharmacists

Dr. Simon Dobson, Pediatric Infectious Disease Specialist

Dr. Jane Gardiner, Pediatric Ophthalmologist

Dr. Dan Metzger, Pediatric Endocrinologist

Luba Scott and Kathleen Gray, Pediatric Renal Nurses

Dr. Chia Wei Teoh, Pediatric Nephrology Fellow

Graphic design

Linda Coe Graphic Design

3RD EDITION • OCTOBER 2016

Page 4: Childhood Nephrotic Syndrome - BC Children’s
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Table of Contents

1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1

2 Diagnosis of nephrotic syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

Swelling (edema) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

Protein losses in the urine (proteinuria) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4

Low blood protein levels (hypoalbuminemia) . . . . . . . . . . . . . . . . . . . . . . . . . 5

3 Types of nephrotic syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6

Steroid sensitive nephrotic syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6

Steroid resistant nephrotic syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6

4 Your child’s first visit to the doctor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8

Checking blood pressure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8

Monitoring kidney function . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8

Testing urine for blood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8

5 Complications of nephrotic syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9

Swelling (edema) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9

Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10

Blood clotting . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10

6 Treatment of nephrotic syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11

Prednisone treatment for initial episode . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11

What will happen after your child starts on prednisonefor the initial episode of nephrotic syndrome? . . . . . . . . . . . . . . . . . . . . . . 13

Prednisone treatment for relapse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14

What will happen after your child starts on prednisonefor a relapse of nephrotic syndrome? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15

Different forms of prednisone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15

Tips on how to give your child prednisone at home . . . . . . . . . . . . . . . 16

Prednisone side effects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17

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7 Diet . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .20

Sodium and fluid . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20

Calcium and vitamin D . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25

Other important diet modifications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25

8 Immunizations and infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27

Live vaccines to avoid . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27

Influenza . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28

Chickenpox (varicella) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28

Pneumococcus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29

9 Ongoing care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30

Follow-up during the first year . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30

Follow-up after the first year . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32

10 Appendix . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35

Commonly used terms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36

Notes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 37

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A PA R E N T ’ S H A N D B O O K O C TO B E R 2 0 1 6 1

IntroductionThe purpose of this handbook is to provide information to parents ofchildren with nephrotic syndrome. We present an organized approachfor the diagnosis and management of this condition. We call thisapproach our nephrotic syndrome clinical pathway. It is the result ofthe work of our group of doctors, students, dietitians, nurses, andfamilies of children with nephrotic syndrome.

This handbook provides general information about the conditionand its treatments. The accompanying Worksheets are practical,easy-to-use, and can be filled in at home. These worksheets areused to keep track of prednisone dosing, urine dipstick results,relapses, possible side effects or complications, and diet intake.Recording your child’s progress and therapy will help us providethe best care for your child.

Our pathway recommendations are based on the best and mostcurrent evidence, our own experience, and the advice of our expertteam and families who care for children with nephrotic syndrome.Welcome to the team!

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2 C H I L D H O O D N E P H R OT I C SY N D R O M E

The Shared Care Renal Program at BC Children’s Hospital

Nephrotic syndrome is one of the most common kidney problems inchildren. In our clinic at BC Children’s Hospital we treat up to 20new patients every year. We continue to follow close to 100 patientsin our renal clinic.

Children with nephrotic syndrome require shared and ongoing care.Families, family doctors, pediatricians, and kidney specialists all playan equally important role. At BC Children’s Hospital, all of our kidneydoctors (nephrologists) are responsible for your child’s care. However,in clinic you may only see a few of them depending on the day of yourappointment. Eventually you will get to know many of our doctorsand they will get to know you. This is particularly useful if you oryour family doctor or pediatrician have to call the hospital after hoursor in an emergency.

When you come to clinic you will meet various members of the renalclinic team as needed (Table 1). We will answer any questions youmay have, make our recommendations, and arrange for your follow-up visit before you leave.

Members of the renal clinic team

TA

BL

E 1

Booking clerk

Nurse’s assistant

Renal clinic nurse

Doctor in training (student, resident, fellow)

Nephrologist

Dietitian

Pharmacist

Social worker

Researcher

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A PA R E N T ’ S H A N D B O O K O C TO B E R 2 0 1 6 3

Diagnosis of nephrotic syndrome Your child has been diagnosed as having nephrotic syndrome becausethey have swelling, protein losses in the urine, and low blood proteinlevels.

Swelling (edema)

Swelling is the most common feature that brings children with nephroticsyndrome to medical attention. It may come and go for weeks priorto the first medical visit. This swelling, particularly around the eyes,is often mistaken for an allergic reaction (Figure 1) and may havebeen treated unsuccessfully with allergy medications. Most childrenand their parents will notice puffy eyelids first, followed by moregeneralized swelling of the feet and legs, and swelling of the belly(abdomen) (Figures 2, 3a, 3b). In severe forms, there is swelling ofthe genitals (scrotum or labia).

The swelling may develop gradually over the course of a few weeksand may shift from one area of the body to another, depending onthe time of day. For example, you may notice more swelling in yourchild’s face early in the morning after sleeping. During the day, theswelling becomes more noticeable in the legs, feet, and ankles.

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Figure 1. Patient has moderate swelling around the eyes.

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4 C H I L D H O O D N E P H R OT I C SY N D R O M E

Figure 4. This is an example of a urine dipstick test. The dipstick at the bottom of thepicture has turned a shade of green after 60 seconds. The green shade would be readas 4+ according to the scale shown, NOT ≥2000 or ≥20 as indicated on the bottle.The + indicators (1+ to 4+) do not actually appear on the bottle.

Protein losses in the urine (proteinuria)

Large losses of protein (albumin) in the urine is an important featureof nephrotic syndrome. This may appear as frothy (foamy) urinein the toilet, but is confirmed by dipping the urine with a dipstick(Figure 4 below and the Urine protein testing at home section of theWorksheets) and a urine protein test sent to the lab.

Figure 2. Patient has mild swelling of the feet and ankles (note the stocking lines).

Figure 3a. Patient has severe swelling of the lower leg that hides ankle definition.

Figure 3b. Gentle pressure with the finger tips leaves a temporary imprint on the lower leg.

2 3b

3a

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A PA R E N T ’ S H A N D B O O K O C TO B E R 2 0 1 6 5

Low blood protein levels (hypoalbuminemia)

Large losses of protein in the urine cause low levels of protein(albumin) in the blood. Low blood protein levels lead to fluid leakingfrom the blood into the body tissues, causing swelling (Figure 5).Low blood protein levels are found by a simple blood test thatmeasures the concentration of albumin.

Figure 5. Diagram of water movement in normal state and in nephrotic syndromeleading to swelling (arrows show water movement).

Normal state

Nephrotic syndrome

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6 C H I L D H O O D N E P H R OT I C SY N D R O M E

Types of nephrotic syndromeThere are different types of childhood nephrotic syndrome. One of thebest ways of classifying nephrotic syndrome is by the response to pred-nisone. Prednisone, is a common “steroid” medicine used in severalchildhood diseases. How a child with nephrotic syndrome respondsto prednisone can often predict the course of illness and the long-term outcome.

Steroid sensitive nephrotic syndrome

Children have steroid sensitive nephrotic syndrome if they respond toprednisone within 6 weeks. This means that by 6 weeks of startingprednisone the swelling has improved and there is no more protein inthe urine. About 80% of children with nephrotic syndrome have steroidsensitive nephrotic syndrome (Pie Chart 1). A response to prednisonemeans your child has nephrotic syndrome likely due to minimal changedisease. With minimal change disease the kidneys look normal whenexamined under a microscope. This is the best type of nephrotic syndrometo have. If your child has steroid sensitive nephrotic syndrome, thereis usually no need for a kidney biopsy as most children who respondto prednisone have minimal change disease (Pie Chart 2).

Steroid resistant nephrotic syndrome

Children have steroid resistant nephrotic syndrome if they do notrespond to prednisone after 6 weeks. This means that by 6 weeksof starting prednisone there is still some swelling and/or protein in theurine. About 20% of children with nephrotic syndrome have steroidresistant nephrotic syndrome (Pie Chart 1). They will require a kidneybiopsy to diagnose the specific type of nephrotic syndrome as theyare less likely to have minimal change disease. Steroid resistantnephrotic syndrome often requires different medicines. Children withsteroid resistant nephrotic syndrome are at higher risk of long-termkidney problems such as reduced kidney function and kidney failure.

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A PA R E N T ’ S H A N D B O O K O C TO B E R 2 0 1 6 7

PIE

CH

AR

T 1

PIE

CH

AR

T 2

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Your child’s first visit to the doctorYour child’s first visit may take place in the doctor’s office, medicalclinic, or emergency room. At the first visit, the doctor will ask youabout your child’s complete medical history, perform a thoroughphysical examination, and order the appropriate laboratory testing. Inparticular they will check your child’s blood pressure, kidney function,and urine. The results of these evaluations will identify any problemsthat need to be dealt with and help predict the long-term response totreatments.

Checking blood pressure

High blood pressure is occasionally seen in children with nephroticsyndrome at their first visit. Sometimes high blood pressure may berelated to the underlying form of nephrotic syndrome. Other factorssuch as pain and anxiety may also cause temporary increases in bloodpressure. It is therefore important to repeat this measurement whenyour child is calm and relaxed. If blood pressure stays high after thefirst visit, treatment may be required.

Monitoring kidney function

Many children with nephrotic syndrome have a temporary decreasein kidney function, which may be due to swelling or being dehydrated.This usually improves once prednisone treatment is started and theswelling decreases, but your child may need to be checked again if thekidney function was found to be abnormal initially. Kidney functioncan be measured with a blood test (creatinine).

Testing urine for blood

At the first visit, the urine test your doctor performs checks for bothblood and protein. If your child has significant amounts of blood inthe urine (hematuria) at the first visit this may require careful attentionsince it is less common in minimal change disease.

8 C H I L D H O O D N E P H R OT I C SY N D R O M E

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A PA R E N T ’ S H A N D B O O K O C TO B E R 2 0 1 6 9

Complications of nephrotic syndrome

Now that your child has been diagnosed with nephrotic syndromeand started on treatment, it is important to watch for complicationsthat might occur either because of the illness itself or because ofthe treatment.

If you think your child needs to be seen or if they are having prob-lems we recommend that you first try to contact your family doctoror pediatrician. If you are not able to reach your doctor, you cancontact the Renal Program at BC Children’s Hospital. During busi-ness hours, please call 604 875 2272 and we will connect you withone of the team members. Outside business hours, please call thehospital switchboard at 604 875 2345 and ask for the kidney doctor(nephrologist) on call.

Swelling (edema)

If severe enough, swelling can lead to breathing difficulties, belly(abdominal) pain, skin changes, and infection. Swelling in the legscan make shoes tight and walking difficult. Severe swelling of the

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Points to remember

Contact your doctor if your child:

1. Has swelling that interferes with normal daily function

or is associated with decreased urine production

2. Develops signs of infection (fever, skin rash, sore throat)

Contact your doctor immediately if your child:

1. Develops a fever AND belly (abdominal) pain

2. Has recently been exposed to chickenpox (varicella) or shingles

3. Develops signs of blood clotting (severe headaches, worse swelling

in one leg, swelling with leg pain, difficulty breathing)

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genitals (scrotum or labia) can be uncomfortable and distressing.Most often attention to sodium and fluid intake will be enough toprevent further swelling while waiting for the prednisone to work(see Section 7 Diet). Severe swelling can affect your child’s kidneyfunction and can lead to a decrease in the amount of urine produced.Sometimes the swelling can be so severe that admission to hospital isneeded for further treatment. If swelling interferes with normal dailyfunctions or there is a drop in urine production while swollen, yourchild should be seen by a doctor.

Infection

Your child’s immune system is weaker than normal because of thelarge losses of protein in the urine with nephrotic syndrome. Themedicines used to treat the nephrotic syndrome including prednisonemake this worse. This puts your child at risk for severe infections. Goodhand hygiene, regular visits to the doctor and dentist, and ensuringyour child’s routine immunizations are up-to-date will help lower theirchance of getting sick. If signs of a possible infection develop, includingfever, skin rash, or sore throat, your child should be seen by a doctor.If a fever AND belly pain develops, your child should be seen by adoctor immediately. If your child has recently been exposed to someonewith active chickenpox (varicella) or shingles, contact your doctorimmediately.

Blood clotting

Your child is at risk of developing clots in blood vessels because ofnephrotic syndrome. This is an uncommon problem but can be serious,often requiring treatment as soon as possible. If it occurs it usuallydoes so around the time of diagnosis, when your child is having arelapse, or if your child has one of the more severe types of nephroticsyndrome. Symptoms of blood clotting include severe headaches,worse swelling in one leg, swelling with leg pain, and difficultybreathing. If you are concerned about signs of blood clotting, yourchild should be seen by a doctor immediately.

10 C H I L D H O O D N E P H R OT I C SY N D R O M E

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A PA R E N T ’ S H A N D B O O K O C TO B E R 2 0 1 6 11

Treatment of nephrotic syndrome

Most children diagnosed with nephrotic syndrome do not requireadmission to hospital. They are typically first seen in the familydoctor’s or pediatrician’s office or the emergency room. The infor-mation provided in this section and the accompanying Worksheetswill help you navigate your child’s course from the first day of beingdiagnosed with nephrotic syndrome and through any relapses thatmight occur in the future.

Prednisone treatment for initial episodeOnce your child is diagnosed with nephrotic syndrome the next step isto start treatment with a medication called prednisone. Prednisoneis the best medication for the initial treatment of nephrotic syndrome.Its benefits are greater than any potential risks. It is very important togive prednisone exactly as it is prescribed. Failure to give prednisonecorrectly may delay your child going into remission, putting them athigher risk of complications. If prednisone is not given as prescribed,your child may be incorrectly diagnosed as steroid resistant(see Section 3 Types of nephrotic syndrome). This may then lead toyour child being placed on other medications for the wrong reason.

We treat the initial or first episode of nephrotic syndrome with 6 weeksof daily prednisone. These 6 weeks of daily prednisone are followedby 6 weeks of alternate day (or every other day) prednisone. The totalduration of initial prednisone treatment adds up to 12 weeks (Figure 6).

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Figure 6. Time course of prednisone treatment for initial episode

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The starting dose of prednisone will be based on your child’s bodysurface area. Body surface area is a measure of your child’s size insquare meters (m2), which is based on height (cm) and weight (kg).A prednisone schedule outlining the starting dose of prednisone andwhen to change from daily to alternate day prednisone will be given toyou. The prednisone dosing for the initial episode is outlined in Table 2.

What is a remission?A remission is defined as 3 days in a row of negative or trace proteinon first morning urine dipstick testing at home. In other words,remission means that for 3 consecutive days urine protein losses stop,swelling decreases, and blood protein levels return to normal. Yourchild has steroid sensitive nephrotic syndrome if, with prednisonetreatment, they go into remission. The majority of children who aregoing to respond to prednisone do so within 2 weeks of treatment.Almost all the children who are going to respond to prednisone doso by 6 weeks of starting treatment. Some older teenagers take longer.Details about monitoring urine protein levels with dipsticks are includedin the Urine protein testing at home section of the Worksheets.

12 C H I L D H O O D N E P H R OT I C SY N D R O M E

Prednisone dosing for your child’sinitial episode of nephrotic syndrome

TA

BL

E 2

6 weeks 60 mg/m2 daily (max 60 mg total)

6 weeks 40 mg/m2 alternate day (max 40 mg total)

STOP

DURATION DOSE BY CHILD’S SIZE (mg/m2)*

*Once daily in the morning on the days prednisone is given

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A PA R E N T ’ S H A N D B O O K O C TO B E R 2 0 1 6 13

What will happen after your child starts on prednisone for theinitial episode of nephrotic syndrome?

After starting prednisone, one of three things will happen:

Your child’s condition will get better: If your child goesinto remission within 4 weeks of initial treatment, youshould continue the prednisone. Your child’s condition willbe reviewed in clinic at the 4-week mark of starting initialtreatment. This is expected in most cases.

Your child’s condition will stay the same: If by 4 weeksafter starting initial treatment your child has not gone intoremission, so long as there are no other major health con-cerns you should continue the prednisone. Your child willbe seen in clinic at the 4-week and 6-week mark of startinginitial treatment to be sure there are no developing concerns.If your child does not go into remission by 6 weeks, they mayrequire a kidney biopsy. This situation occurs less frequently.

Your child’s condition will get worse: If while waiting to re-spond your child becomes more swollen or experiences anyof the other complications (see Section 5 Complications ofnephrotic syndrome), you should continue the prednisone andcontact your doctor for further advice. This situation alsooccurs less frequently.

What is a relapse?A relapse is defined as 3 days in a row of 3+ or 4+ protein on firstmorning urine dipstick testing at home — after previously being inremission (see What is a remission?).

Most children with nephrotic syndrome have relapses that requirerepeated prednisone treatment, so do not be alarmed or discouragedif this happens to your child. Approximately 90% of children withnephrotic syndrome will have a relapse. Most relapses are triggeredby an infection, such as a cold or flu. Even very minor infections,dental cavities, or bug bites can sometimes trigger a relapse. A relapsewill often require another course of prednisone treatment. Your childmay not have swelling at the time a relapse is diagnosed; however,if left untreated, they will often develop swelling.

If a relapse happens you should contact either your family doctor orpediatrician. If you are having problems contacting them, give us a call.

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14 C H I L D H O O D N E P H R OT I C SY N D R O M E

Figure 7. Time course of prednisone treatment for relapses

If your child has multiple relapses requiring repeated courses ofprednisone they may need other forms of treatment.

Prednisone treatment for relapse

It is very likely that your child will have one or more relapses aftertheir initial episode. Unfortunately we cannot predict the timing orfrequency of relapses. Urine dipsticks are used to confirm a relapseat home (Figure 4). Relapses will be treated with daily prednisoneuntil your child’s first morning dipsticks are negative or trace for3 days in a row (remission). Once back in remission, the prednisonedose will be reduced to alternate day treatment for 2 weeks as outlinedin Figure 7 and Table 3. The treatment of relapses is shorter thanthe initial treatment.

Prednisone dosing for your child’srelapse of nephrotic syndrome

TA

BL

E 3

DURATION DOSE BY CHILD’S SIZE (mg/m2)*

Until 60 mg/m2 daily (max 60 mg)remission

2 weeks 40 mg/m2 alternate day (max 40 mg)

STOP

*Once daily in the morning on the days prednisone is given

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A PA R E N T ’ S H A N D B O O K O C TO B E R 2 0 1 6 15

What will happen after your child starts on prednisone fora relapse of nephrotic syndrome?

After starting prednisone, one of three things will happen:

Your child’s condition will get better: If your child goesinto remission within the first 2 weeks of relapse treatment,you should contact your doctor to discuss the details ofdecreasing the prednisone. You do not have to be seen inclinic at this point.

Your child’s condition will stay the same: If by 2 weeks afterstarting relapse treatment your child has not gone into remis-sion, you should contact your doctor. Your child will be seenin clinic at the 4-week mark of starting relapse treatment tobe sure there are no concerns.

Your child’s condition will get worse: If after starting relapsetreatment your child becomes more swollen or develops anyof the other complications (see Section 5 Complications ofnephrotic sydnrome), you should contact your doctor as yourchild may need to be seen promptly.

Different forms of prednisone

Prednisone can be given in both tablet and liquid form. Tablets come indifferent strengths (1, 5 and 50 mg). We will use whatever tablets aremost convenient for your child to help make taking prednisone quickand easy for them. The 5 mg tablets are generally small and easier toswallow (Figure 8).

The liquid form is called prednisone or prednisoLONE. The concen-tration of liquid prednisone can vary from 1 mg/mL to 5 mg/mLdepending on the pharmacy, so it is important to be aware of theexact concentration you are provided.

Figure 8. 5 mg prednisone tablets areclose in size to the end of a pencil.

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Tips on how to give your child prednisone at home

Tablet prednisone (50 mg, 5 mg, 1 mg)

Have your child swallow the tablet(s) quickly. If it sits on the tongueit begins to dissolve and has a bitter taste. You may crush the tablet(s)and give with a small spoonful of soft food such as jam, ice cream,yogurt, pudding, jello, custard or similar. Put the powdered tabletonto the food, and ensure the entire amount is swallowed.

Liquid prednisone

Shake well before measuring the dose in the plastic syringe provided.Give the prednisone as is (do NOT dilute). Place the oral syringeinto your child’s cheek toward the back of the mouth and gentlysqueeze the prednisone into their cheek. Blow on your child’s faceas it stimulates them to swallow. You may need to give the doseof prednisone in repeated smaller amounts over 10 or 15 minutes,especially if the amount is large. After the prednisone, you may giveyour child water or a strong tasting food or drink, but NOT theirfavorite as they may learn to dislike it. You may need to swaddleyour child in a towel or hug them tightly to give this medicine ifthey are resistant. You may even need to hold your child’s nose tomake them open their mouth.

Points to remember

DO give prednisone with food. Prednisone can cause upset stomach.

This can be reduced by giving your child their prednisone with food.

Giving prednisone in the morning with breakfast is recommended.

DO NOT try to hide prednisone in food, like a bowl of cereal or other food.

Your child may not finish the food, they may develop a distrust of you

and of food, or they may start to hate a previously loved food or drink.

DO NOT dilute the prednisone in a glass of liquid. Your child may not

drink the whole amount and would not get the full dose.

DO NOT freeze liquid prednisone. Freezing may weaken the prednisone

so that it is not effective.

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Prednisone side effects

Weight gain

Prednisone often causes an increase in appetite. Because of this, it iscommon for children on prednisone to gain extra weight. Makinghealthy food choices can help minimize extra weight gain (see Section 7Diet). Your child’s appetite will likely normalize once they are off dailyprednisone. Being physically active will also help minimize extra weightgain. All children taking prednisone are encouraged to remain phys-ically active.

Stomach pain

While on daily prednisone, yourchild may experience stomach pain(gastritis) or heart burn (esophagitis).These side effects get better as theprednisone dose is decreased. If thestomach pain or heartburn continue,let your family doctor or pediatricianknow as it can be treated with med-ications such as Ranitidine.

High blood pressure

While on daily prednisone, yourchild may experience high bloodpressure. The most common symp-toms of high blood pressure areheadache and sometimes dizzinessand shortness of breath. However,because many children with highblood pressure do not have anysymptoms at all it is important torecord the blood pressure at eachvisit to the doctor. If necessary,high blood pressure will be treated. If any symptoms of high bloodpressure occur, let your family doctor or pediatrician know.

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Eye problems

Long-term use of prednisone can cause eyeproblems. The two most common eye problemsseen are cataracts and glaucoma. Cataractsmay present as blurred vision and glaucomamay present as eye pain. Symptoms areinfrequently seen with either condition.Your child will be referred for a check-up with an eye doctor(ophthalmologist) during their initial prednisone treatment and thenlater if needed.

Bone problems

Prednisone may cause bone problems, including decreased bone den-sity (osteopenia) and increased risk of bone fractures. These are moreof a concern in children who require repeated courses of prednisoneover time due to frequent relapses of their nephrotic syndrome. It isimportant to meet Health Canada’s recommended daily intake forcalcium and vitamin D. Staying activeand maintaining a healthy weight arealso important in helping reduce the riskof bone disease and fractures.

To ensure children with nephroticsyndrome get enough calcium and vita-min D, we recommend supplementing500 mg elemental calcium and 800–1000 IU vitamin D on top oftheir diet (see Section 7 Diet). If your child develops bone pain or hasa bone fracture, contact your family doctor or pediatrician.

Cosmetic changes

While on prednisone, your child may experience extra hair growth ontheir face and body (hirsutism), hair thinning, acne, skin streaks (striae),an enlarged belly, or a moon-shaped face (Cushingoid facies). These arewell described side effects of prednisone that will get better and usuallydisappear as the prednisone dose is decreased.

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Behaviour changes

While on prednisone, your child may experience mood changes,difficulty sleeping, or hyperactivity. These are well described sideeffects of prednisone that will get better and usually disappear asthe prednisone dose is decreased.

Low adrenal gland function

The adrenal glands help the body respond to stress, like illnesses oraccidents. The use of prednisone can lower the function of the adrenalglands and make it harder for the body to respond to stressful events.The most common symptoms of low adrenal gland function aretiredness (fatigue), low appetite, weight loss, and muscle weakness.These side effects usually get better once your child is completely offprednisone for a period of time. If they do not get better then furthertreatment is sometimes needed. Your doctor may recommend certaintests to monitor for this.

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DietDiet plays an important role in the management of your child'snephrotic syndrome. Our recommendations for your child’s dietare particularly important around the time of diagnosis and duringrelapses. Making healthy food choices will help to reduce swellingand control blood pressure, prevent excessive weight gain duringprednisone treatment, and keep bones healthy. Your child’s diet will bereviewed at initial diagnosis, then again at the 4-week and 12-monthclinic visits and more frequently as needed. Use the 3-day FoodIntake Record sheets provided in the Worksheets to track your child’sintake at 4-weeks and 12-months.

The dietitians at BC Children’s Hospital are available to answer anyof your questions. In situations where a dietitian is not available, youcan also call HealthLink BC (dial 8-1-1).

Sodium and FluidWe have two approaches to help reduce swelling associated withnephrotic syndrome. First, we recommend a diet limited in sodium forthe duration of prednisone treatment. Second, we also recommenda daily fluid limit until your child is in remission. Reducing theamount of sodium in your child’s diet makes your child less thirsty,making it easier to stay within their prescribed fluid allowance. Ourrecommendations for the amounts of sodium and fluid are based onyour child’s gender and weight.

Table 4 shows the maximum amount of sodium in milligrams (mg)your child should consume each day while they are taking prednisone.Table 5 shows the maximum amount of fluid in milliliters (mL) yourchild should consume each day until remission.

For example, if your daughter weighs 20 kg (44 pounds) and shehas a relapse of her nephrotic syndrome, we recommend maximum1,300 mg of sodium per day for the time she is taking prednisone

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(Table 4) and 800 mL of fluids per day until she goes into remission(Table 5). Careful attention to the sodium content of foods you areoffering, as well as dividing the fluid allowance across the day will helpyour child to adhere to the recommended sodium and fluid limits.

Decreasing sodium and fluids beyond these recommendations is notnecessary. Recommendations for your child’s diet will be reviewedwith you at your visits to the clinic.

Recommended maximum dailysodium intake while on prednisone

TA

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BOYS GIRLS

10–19 800 800

20–29 1300 1300

30–39 1600 1400

40–49 1700 1600

50–59 2000 1700

60–69 2300 1800

70 and 2400 1900above

WEIGHT (kg) SODIUM INTAKE (mg/day)*

*While your child is on prednisone

Recommended maximum dailyfluid intake until remission

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10–19 500

20–29 750

30–39 900

40–49 1000

50–59 1100

60–69 1200

70and 1300 above

WEIGHT (kg) FLUID INTAKE (mL/day)*

* Provide the followingfoods with caution asthey are made up of alarge amount of water:pudding, yogurt, jello,popsicles, watermelonand pureed fruits

*Until your child is in remission

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To help achieve the recommendations for sodium and fluid, extraattention to your child’s diet will be needed. Choosing foods thatfit can be a challenge! Here are some tips to get you started:

What is sodium and where is it found?

Sodium is a mineral found in table salt, baking soda and many foodpreservatives. Small amounts of sodium occur naturally in foodswhile foods that have been processed may have high amounts ofsodium. About 80% of the sodium that Canadians consume comesfrom salt that has been added to foods during processing.

Table 6 illustrates how the sodium content of foods in their naturalform increases as they are processed.

Cucumber 7 mg

Tomato1 small 14 mg

Chicken½ breast 69 mg

Pork3 ounces 59 mg

Roast beef3 ounces 54 mg

Cheddar cheese1 ounce 173 mg

Cooked oatmeal1 cup <1 mgShredded Wheat1 round <1 mgSteamed rice/cooked pasta1 cup <1 mg

Olive oil1 tablespoon <1 mgLemon juice1 tablespoon 1 mgUnsalted butter/margarine1 tablespoon 2/0 mg

Cucumber withRanch dressing 145 mg

Tomato ketchup2 packages 140 mg

Chicken nuggets + sauce(4 nuggets) 670 mg

Bacon4 slices 548 mg

Hot dog weiner1 regular 487 mg

Processed cheese slice1 slice 390 mg

Bread2 slices 298 mg

Rice Krispies®1 cup 298 mg

Salted butter/margarine1 tablespoon 81/70 mg

Dill pickle1 medium 928 mg

Tomato sauce½ cup 640 mg

Chicken luncheon meat3 ounces 1059 mg

Ham3 ounces 1,114 mg

Pepperoni3 ounces 892 mg

Cheese spread2 tablespoons 540 mg

Instant noodles1 serving 2,200 mg

Soy sauce1 tablespoon 1,029 mg

Salt1 teaspoon 2,325 mg

HEALTHY CHOICE LESS HEALTHY CHOICE UNHEALTHY CHOICE

Sodium content of foods

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As you move from left to right in Table 6 you will notice that foodshave increasing amounts of sodium. The preferred foods would bethose on the left in the green boxes as they have less sodium. Foodslisted on the far right in the red boxes contain the most amount ofsodium and should be avoided.

Sample menu

The menu plan in Table 7 was created for a 10-year-old child beingtreated with prednisone for nephrotic syndrome. The menu suggestionsare low in sodium, relatively low in fat, and contain adequate calcium,all important recommendations for children with nephrotic syndrome.A diet low in sodium can be healthy and enjoyable for ALL familymembers.

MEAL LOW SODIUM SUGGESTION

Breakfast

Morning snack

Lunch

Afternoon snack

Dinner

Evening snack

1 cup Shredded Wheat/Mini Wheats®1 cup 1% milk (½ on cereal + ½ to drink) 1 medium banana

1 orange

1 jam sandwich 2 graham crackers½ cup yogurt1 small apple

3 cups unsalted popcornCarrot sticks

2 oz chicken breast1 cup steamed rice1 cup green salad1 tablespoon oil + balsamic vinaigrette½ cup steamed broccoli½ cup corn niblets 1 cup of 1% milk

½ cup applesauce1 cup 1% milk

Sample menu

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In order to choose the right foods for your child, it is important toread and understand food labels. Be sure to check labels for thesodium content of any packaged foods. See Reading food labelsfor some additional information to help you determine how muchsodium is in the packaged foods you choose.

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Reading food labels

In Canada, packaged foods are required to have a Nutrition Factsbox on the label. By law, sodium is one of 13 items that is requiredto be reported on the package label. When reading labels, followthese simple instructions to determine whether the food item isappropriate for your child with nephrotic syndrome:

1 Note the number of calories provided by the serving size listed.

2 Note the number of mg of sodium in the serving.

3 Choose food items that have LESS sodium mgs than calories.

Figure 9 and Figure 10 are two examples where reading the labelshelps make the right choice:

Figure 9. This nutrition label showsthe food item provides 80 calorieswith 0 mg of sodium. The number ofmg of sodium is less than the numbercalories, therefore, an appropriate low-sodium choice.

Figure 10. This nutrition label showsa food that contains 170 calories with620 mg of sodium. The number ofmg of sodium is nearly four timesthe number of calories, and is NOTan appropriate low-sodium choice.

Nutrition FactsValeur nutritivePer 1 tray (212 g)/Pour 1 plat (212 g)

Amount % Daily ValueTeneur % valeur quotidienne

Calories/Calories 170

Fat/Lipides 2.5 g 4%

Saturated/Saturés 0.5 g 3%+ Trans/Trans 0 g

Cholesterol/Cholestérol 25 mg 8%

Sodium/Sodium 620 mg 26%

Carbohydrate/Glucides 25 g 8%

Fibre/Fibres 2 g 8%

Sugars/Sucres 2 g

Protein/Protéines 12 g

Vitamin A/Vitamine A 4%

Vitamin C/Vitamine C 15%

Calcium/Calcium 2%

Iron/Fer 8%

Nutrition FactsPer 125 mL (87 g)*

Amount % Daily Value*

Calories 80

Fat 0.5 g 1%

Saturated 0 g 0%+ Trans 0 g

Cholesterol 0 mg

Sodium 0 mg 0%

Carbohydrate 18 g 6%

Fibre 2 g 8%

Sugars 2 g

Protein 3 g

Vitamin A 2% Vitamin C 10%

Calcium 0% Iron 2%

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Calcium and vitamin D

An important side effect of nephrotic syndrome and being treatedwith prednisone is long-term bone problems. We therefore recommenda minimum daily calcium and vitamin D intake, which depending onhow much prednisone your child needs, may vary from time to time(see Section 6 Treatment of nephrotic syndrome). To ensure childrenwith nephrotic syndrome get enough calcium and vitamin D, werecommend supplementing 500 mg elemental calcium and 800–1000 IU vitamin D on top of their diet while they are on prednisone.

What foods provide calcium?

Calcium is found in many foods. Your child can get recommendedamounts of calcium by eating a variety of foods, including thefollowing: milk and milk alternatives such as yogurt, cheese andfortified plant-based beverages (such as fortified soy beverages);dark green vegetables such as broccoli, kale and spinach; fish withsoft bones that are eaten, such as canned salmon or sardines.

What foods provide vitamin D?

The major sources of vitamin D are vitamin D-fortified foods, includingcow’s milk and margarine. Goat’s milk, plant-based beverages such assoy, and some calcium-fortified orange juices may not be fortified withvitamin D. Cheese and yogurt can be made with vitamin D-fortifiedmilk, but the final product does not contain as much vitamin D asfluid milk alone. The only natural sources of vitamin D in the Canadianfood supply are fatty fish and egg yolks.

Other important diet modifications

Prednisone is an important medication in the treatment of nephroticsyndrome. This medication can have side effects such as an increasedappetite which may lead to weight gain and it can also cause highblood sugar. Limiting intake of sugary foods and choosing the righttype and amount of fat will help to reduce weight gain.

Protein

Even though your child may be losing protein in the urine, we do notrecommend increasing dietary protein.

Sugars

Limit the sugar your child eats by avoiding fruit juice or limiting intaketo a maximum of 1 cup/day. Avoid beverages with added sugar suchas fruit drinks, soft drinks, pop, fruit punch, iced tea and lemonade.

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Encourage your child to eat fruit and to drink water or milk as pre-scribed. Limit candies, cookies, ice cream and pastries.

Fats

Strategies to help limit excessive weight gain include limiting foodswith saturated fats and cholesterol, cooking with oils that are liquidat room temperature, and using soft unsalted margarines in place ofbutter or stick margarines. Serve low-fat or nonfat milk and yogurts.Cut the visible fat off of meat and poultry. When choosing packagedfoods, look for foods made without saturated or trans fats.

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Immunizations and infectionsBecause of your child’s nephrotic syndrome and the prednisone usedto treat this condition, your child will be unable to fight infectionsproperly. They will be at greater risk of getting sick. Viral infections,including the seasonal flu and chickenpox (varicella) may be severe.Your child is also at higher risk for developing bacterial infections ofthe belly (peritonitis), skin (cellulitis), and lungs (pneumonia).

Good hand hygiene, regular visits to the doctor and dentist, andensuring your child’s immunizations are up-to-date will help lowertheir chance of getting sick. If possible, they should also minimizecontact with noticeably sick people. However, drastic changes totheir normal day-to-day routine are not recommended. With respon-sible monitoring, they can still go to school, join activities, and playwith friends. If you have any questions or concerns about infections,contact your doctor.

Live vaccines to avoid

Live vaccines are vaccines that contain a weak but living form of virus.Since children with nephrotic syndrome are at greater risk of gettingsick, live vaccines should be avoided while they are in relapse and/oron prednisone. Live vaccines to avoid include MMR (Measles, Mumps,Rubella), varicella (chickenpox), intranasal influenza (FluMist®),

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Points to remember

1. Contact your doctor if you have any concerns about infection.

2. Ensure your child’s immunizations are up-to-date.

3. Avoid live vaccines while your child is in relapse and/or is on

prednisone.

4. Bring your child’s immunization records to your clinic visit and

each time they get a new vaccination.

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yellow fever, and BCG (Bacillus Calmette-Guerin). Note that intranasalinfluenza, yellow fever, and BCG are not part of the routine BritishColumbia immunization schedule (www.immunizebc.ca).

Influenza

Your child can have a severe course of influenza A or seasonal fluinfection. These infections can often trigger a relapse of nephroticsyndrome. We recommend annual influenza immunizations regardlessof whether your child is in remission, in relapse, or if they are takingprednisone. To further reduce the risk of infection to your child wealso recommend immunizing all family members during flu season,which typically starts in the fall.

A live influenza vaccine is now available as a nasal spray (FluMist®).However it may not be as effective and we do not recommend its use.

Chickenpox (varicella)

Typical chickenpox starts with a fever followed by a red rash thatoften develops into little blisters (Figures 11a, 11b). The chickenpoxvirus spreads through the air (by coughing and sneezing) and bydirect contact with mucus, saliva, or fluid from the blisters. Chick-enpox is contagious from about 2 days before the rash appears anduntil all the blisters are crusted over.

Your child should be immunized with the varicella vaccination. InBritish Columbia, chickenpox immunization is given at 12 monthsand again at 4–6 years of age. We consider children to be likelyimmunized if they had at least one chickenpox immunization. Severechickenpox infections are a real risk for all non-immunized children

Figure 11a. Patient with varicella blisterson the neck resembling “dew drops onrose petals.”

Figure 11b. Patient with varicella blisterson the shoulder.

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with nephrotic syndrome. If your child has not been fully immunizedfor chickenpox (has not had 2 immunizations), they should get theirvaccination once they are in remission AND off prednisone for morethan 4 weeks. To further reduce the risk of infection to your child,we recommend that all the members of your family also be immunizedif they have not had chickenpox or have not been immunized.

If your child has not had chickenpox, has not been fully immunizedAND is exposed to anyone who has chickenpox or shingles, contactyour doctor immediately. If your child develops signs of chickenpox,even if they have been previously immunized, contact your doctorimmediately.

Pneumococcus

Your child is at risk for developing severe infections from a bacteriumcalled pneumococcus. These infections include severe belly infections(peritonitis), severe lung infections (pneumonia), and severe skin infec-tions (cellulitis). Therefore it is important that your child receives alltheir pneumococcal immunizations according to the provincial schedule(www.immunizebc.ca). In British Columbia, standard pneumococcalimmunizations are given at 2 months, 4 months, and 12 months ofage. Most children who present with nephrotic syndrome are fullyimmunized by the time that they are diagnosed. However, due to thehigher risk of infection with specific types of pneumococcus that arenot covered with the standard schedule, we recommend an additionalone time pneumococcal vaccination (PneumovaxTM) that can bearranged through the family physician or closest public health clinic.

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SECTIO

N9Ongoing care

Follow-up during the first year

After your child’s initial diagnosis they will need to be followed closelyover the first year. The goals of follow-up during this time are todocument their response to prednisone and to look for complicationsof nephrotic syndrome or for possible side effects from prednisonetreatment. We recommend clinic visits within 1 week from initialdiagnosis, then at 4 weeks, 12 weeks, 6 months, 9 months, and12 months after diagnosis (see Table 8 Schedule of ongoing care).

The 1-week clinic visit will be used for teaching. We want to makesure that your child is properly started on prednisone after diagnosisand that you are comfortable with home monitoring, such as usingdipsticks and knowing when to call your doctor about complications.

The 4-week visit will be used for more teaching and to check theresponse to prednisone treatment. Your child should also have a checkup with an eye doctor (ophthalmologist) around this time. This is tocheck for possible eye problems related to prednisone. Your doctormay also request other tests for your child at this time. These tests maybe done to review bone health or adrenal gland function, as needed.

An extra 6-week visit will only be necessary if your child has still notyet responded to prednisone.

At 12 weeks, if all has gone according to plan, your child will be offprednisone. The 12-week visit will focus on monitoring the effects ofprednisone treatment.

At 6, 9, and 12 months, visits will focus on your child’s growth andthe education around treatment and management of future relapses.More visits may be required based on your child’s individual needs.

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At all visits, general good health practices such as routine dental check-ups and up-to-date immunizations will be reviewed. A complicatedcourse or unusual presentation may require more attention, includingongoing support from our dietitian or other health care workers.

Table 8 summarizes when the visits in the first year will be scheduledand what will be reviewed.

No regular blood testing needed

Confirm and explain diagnosis �

History

• History of swelling �

• Review immunization status � � � � � � �

• Document date and time to remission � �

• Review Handbook Worksheets � � � � � �

• Review prednisone schedule � � � � � �

• Review medication history � � � � � � �

Physical examination

• Blood pressure, heart rate � � � � � � �and temperature

• Record growth (height, weight) � � � � � � �

• Check for swelling � � � � � � �

• Other signs of prednisone � � � � � �side effects (eyes, bone, skin)

Lab testing

• CBC, albumin, urea, creatinine, �Na+, K+, Cl–, HCO3

• Urinalysis, microscopy, and PCR � � � � � � �

Monitoring

• Review fluid restriction � �

• Review dietary recommendations � � � �

• Review home monitoring � � � � � � �(urine dipsticks)

• Eye clinic evaluation �

• Family given handbook & worksheets �

• Schedule follow-up appointment � � � � � � �

TEST/REVIEW

CLINIC VISIT

0 w* 4 w 6 w** 12 w 6 m 9 m 12 m

* Visit at which prednisone is started. A subsequent clinic visit within 1 week islikely needed to complete all necessary tests and teaching

** A six-week visit is required if your child is not in remission at 4 weeks

Schedule of ongoing care

TABLE 8

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Follow-up after the first year

After the first year the frequency of your child's visits will dependon how well they are doing and how often they relapse. Most ofthe time relapses can be dealt with over the phone with your familydoctor or pediatrician. No extra lab tests will be needed. If your childhas complications during a future relapse, such as severe swelling orno response to prednisone, you may need to return to the clinic formore treatment.

If your child continues to have relapses of their nephrotic syndrome,they will need to be seen more frequently to be sure they have respondedproperly to treatment and that they have not developed side effects oftreatment. Regardless of course, most children will need to be seen bya kidney doctor (nephrologist) at least once a year.

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Appendix

Commonly used terms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36

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A P P E N D I X

Commonly used terms

Albumin Protein made by the liver that makes up nearly half ofthe total blood proteins

Ascites Accumulation of fluid in the abdominal cavity

Biopsy Sample of body tissue or cells for testing includingmicroscopic review

Cellulitis Infection of the skin, causing localized swelling, rednessand pain

Childhood onset Nephrotic syndrome presenting between 1 and 18nephrotic syndrome years of age

Creatinine Waste product of muscle that is filtered by the kidneys(used as a marker for kidney function)

Frequent relapsing Nephrotic syndrome with 4 or more relapses in anynephrotic syndrome 12 months or 2 or more relapses in the first 6 months

after presentation

Glomerulus Filter unit of the kidney

Hematuria Blood in the urine — may be either visible to the eye(macroscopic) or only under the microscope (microscopic)

Hypertension High blood pressure

Immunocompromised State of decreased resistance to infections because ofdisease or the use of certain drugs

Minimal change disease The most common type of nephrotic syndrome in childrenwho respond to prednisone. It is characterized by normallooking kidney filters (glomeruli) when examined under amicroscope

Nephrologist Doctor specializing in kidney disease

Nephrotic syndrome Clinical diagnosis consisting of swelling, low bloodalbumin and heavy protein losses in the urine

Peritonitis Infection of the layers lining the abdominal cavity

Proteinuria Loss of proteins in the urine, usually discovered by a urinedipstick

Relapse Three days in a row of 3+ or 4+ protein on urine dipstick

Remission/response Three days in a row of negative or trace protein on urinedipstick

Steroid dependent Nephrotic syndrome responsive to prednisone, but relapsesnephrotic syndrome occur while on steroids or within 14 days after stopping

steroids

Steroid resistant Nephrotic syndrome not responding to prednisone afternephrotic syndrome 6 weeks of daily prednisone therapy

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Notes

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Notes

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British Columbia Children’s Hospital4480 Oak StreetVancouver, BC V6H 3V4

Hospital SwitchboardTel 604 875 2345Toll free in BC 1 888 300 3088

Division of NephrologyTel 604 875 2272